Nephrotic Syndrome

A 6-year-old boy is brought to the emergency department by his mother due to swelling around his eyes and legs. The mother reports that the patient recently recovered from an upper respiratory tract infection. Physical exam is significant for periorbital and lower extremity edema. Laboratory testing is significant for hypoalbuminemia and normal complement levels. Urinalysis demonstrates 4+ protein. A presumptive diagnosis of minimal change disease is made and the patient is started on steroid therapy.

Introduction

Clinical definition

a type of kidney disease that results in proteinuria, peripheral edema, hyperlipidemia, and hypoalbuminemia

Epidemiology

incidence

annually there are 3 cases per 100,000 adults

Etiology

primary glomerular disease

focal segmental glomerulosclerosis

membranous nephropathy

minimal change disease

secondary causes

diabetic nephropathy

systemic lupus erythematosus

amyloidosis

Pathogenesis

the glomerulus becomes permeable to large molecules (e.g., albumin)

this loss of albumin (proteinuria) results in hypoalbuminemia and edema

associated with a hypercoagulable state

pathophysiology unclear but may be due to loss of antithrombin and plasminogen proteins

(M1.RL.88)
A 57-year-old male with diabetes mellitus type II presents for a routine check-up. His blood glucose levels have been inconsistently controlled with medications and diet since his diagnosis 3 years ago. At this current visit, urinalysis demonstrates albumin levels of 250 mg/day. All prior urinalyses have shown albumin levels below 20 mg/day. At this point in the progression of the patient’s disease, which of the following is the most likely finding seen on kidney biopsy?
Review Topic

(M1.RL.84)
A 6-year-old girl presents to your clinic two weeks after receiving a routine immunization in preparation for a trip overseas. Periorbital edema is present on exam, and 24 hour urine collection shows excretion of 4.3 grams of protein/day. Which pathological change would likely be seen on microscopy?Review Topic

QID:
101038

1

Linear IgG deposition on light microscopy

6%

(2/36)

2

IgA-immune complexes in the mesangium on electron microscopy

14%

(5/36)

3

“Tram-track” appearance on light microscopy

6%

(2/36)

4

Subepithelial deposits with “spike and dome” appearance on electron microscopy

(M1.RL.70)
A 7-year-old boy suffers from generalized edema. Urine protein excretion is 5.2 g over 24 hours, and serum analysis reveals hyperlipidemia. The patient responds to treatment with prednisone, and, 8 weeks later, his urine does not contain measurable protein. If a kidney biopsy had been performed while the patient’s condition was pathologic, which of the following would you expect to find upon glomerular electron microscopy?
Review Topic

(M1.RL.59)
A 52-year-old African American male with a history of HIV and obesity presents to his primary care physician because of recent changes to his health. He states that while he has been compliant on his HIV medication, he has noticed recent fatigue and increased swelling in his face and legs. A routine urinalysis demonstrates proteinuria and fatty casts. Based on these findings, the physician decided to proceed with a kidney biopsy. Which of the following figures most likely represents the findings of this patient's kidney biopsy?
Review Topic

(M1.RL.74)
A 57-year-old female visits her primary care physician with 2+ pitting edema in her legs. She takes no medications and does not use alcohol, tobacco, or illicit drugs. 4.5 grams of protein are collected during 24-hour urine excretion. A kidney biopsy is obtained. Examination with light microscopy shows diffuse thickening of the glomerular basement membrane. Electron microscopy shows subepithelial spike and dome deposits. Which of the following is the most likely diagnosis:
Review Topic

(M1.RL.24)
An 11-year-old boy presents to your office with pitting edema and proteinuria exceeding 3.5g in 24 hours. You suspect that this patient has experienced a loss of polyanions in his glomerular basement membranes. Which of the following findings would confirm your diagnosis?
Review Topic

(M1.RL.19)
A 55-year-old African American male presents to his primary care physician with complaints of persistent back pain and fatigue over 12 months. Physical examination reveals a blood pressure of 190/150 mm Hg, and laboratory tests reveal hyperlipidemia and a serum creatinine level of 3.0 mg/dL. 4.5 g of protein are excreted in the urine over 24 hours. Renal biopsy shows eosinophilic, acellular material in the glomerular tuft and capillary walls that display apple green-colored birefringence in polarized light upon Congo red tissue staining. The patient most likely suffers from which of the following:
Review Topic

(M1.RL.8)
A 5-year-old female presents to your office with periorbital edema and proteinuria. Her mother reports that she "just got over a cold" last week. On examination, you note a pulse rate of 70/minute and a blood pressure of 95/53. Which protein(s) would likely be found in large amounts in this patient's urine?
Review Topic

(M1.RL.81)
A 67-year-old man presents to your office with a chief complaint of constipation and many other perturbing minor medical concerns. He reports tiring easily, which he attributes to old age and years of persistent pain in his back and ribs. A complete blood count shows low hemoglobin and elevated serum creatinine. A peripheral blood smear shows stacks of red blood cells among other findings, and serum electropheresis reveals an abnormal concentration of protein resulting in a spike. Which of the following additional findings would you expect to see in this patient?
Review Topic